The spectrum of spitzoid tumours: A clinical study

This study explores the relationship between different types of spitzoid tumours, spindle cell naevus of Reed and spitzoid melanomas. Clinical and histopathological data were retrospectively reviewed from our hospital database in Cambridge from January 2006 to July 2009. Clinical images, where available, were recorded. Search headings from our pathology database included ‘spitzoid tumours’, ‘Spitz naevi’, ‘atypical spitzoid tumours’, spitzoid tumours of uncertain malignant potential (‘STUMP’), ‘spindle cell naevus of Reed’ and ‘spitzoid melanomas’. The total number of spitzoid tumours was 118 comprising Spitz naevi (72), atypical spitzoid tumours (30), spitzoid melanomas (eight), and other naevi with spitzoid features (eight). In total, 60% of Spitz naevi were diagnosed clinically and 50% reported a history of change with spitzoid melanoma, compared with 32% with Spitz naevi. In all, 60% of Spitz naevi and atypical spitzoid tumours were pigmented in contrast with spitzoid melanomas (83%). Variegated pigmentation was found in 20% of Spitz naevi and atypical spitzoid tumours, however, no spitzoid melanomas had mixed pigmentation. There were 30 atypical spitzoid tumours (9 M : 21 F); 16 occurred on the lower limbs, peaking in the 20–30‐years age group. There were eight patients with spitzoid melanomas with a 7:1 F : M ratio, 50% of which were diagnosed clinically. Of the 34 spindle cell naevus of Reed (10 M : 24 F), 31 were misdiagnosed, most commonly as melanoma. Reed naevi peaked in the 30–40 year age group and on the upper limbs and lower limbs in the 20–30‐years age group. In summary, age and sex appeared helpful in distinguishing benign from malignant spitzoid tumours, however history was less discriminatory. Spitzoid melanomas, most of which were pigmented occurred more commonly in females. Atypical spitzoid tumours were more common in females and pathologists favoured malignancy in this group beyond 20 years of age.     

Pigmented spindle cell naevus

We report 22 cases of pigmented spindle cell naevus (PSCN). The usual appearance of these naevi is that of a heavily pigmented papule found mostly on the legs of young patients. Histologically, PSCN was characterized by symmetrical proliferation of spindle-shaped pigmented melanocytes grouped in large junctional nests. Pagetoid spread of single cells in the overlying epidermis was frequently found. In our opinion, PSCN is a distinctive benign acquired melanocytic naevus that in the past has been frequently misdiagnosed as atypical Spitz naevi or malignant melanoma.     

Benign melanocytic proliferative nodule within a congenital naevus

A neonate presented with a deeply pigmented papule within a medium-sized congenital naevus. Histologically, this proved to be a benign proliferative nodule in a congenital naevus. This case is presented to highlight the occurrence of this lesion, the main differential of which is the rare entity of true congenital melanoma.     

Pigmented spindle cell nevus and pigmented Spitz nevus--clinical and histopathological study on pigmented Spitz nevus, and its differentiation from early melanoma by fluorescence method and measurement of 5-S-CD level in the lesion

On the basis of clinical and histopathological studies on 17 patients who had been diagnosed as having pigmented Spitz nevus (PSN), pigmented spindle cell nevus (PSCN) was surmised to be a type of pigmented Spitz nevus. In order to distinguish pigmented spindle cell nevus and pigmented Spitz nevus from early melanoma, 5 PSCN cases and 12 PSN cases were analyzed by the fine-needle aspiration fluorescence method, touch fluorescence method and measurement of the 5-S-CD level in the lesion. With the touch fluorescence method, fluorescent tumor cells were detected in one case of PSN. With the fine-needle aspiration fluorescence method, fluorescent tumor cells were detected in one PSCN case and 2 PSN cases. In comparison with fluorescent melanoma cells, the detected fluorescent tumor cells were smaller in size and number and resembled melanocytes. The 5-S-CD level in the lesion was 50 ng/mg or less in all cases, whereas the level in melanoma is known to be a high 100 ng/mg or more. In the final analysis, measurement of the 5-S-CD level in the lesion was concluded to have the greatest utility for differential diagnosis of pigmented spindle cell nevus and pigmented Spitz nevus from early melanoma.     

Meyerson's naevus: a clinical and histopathological study of 11 cases

We undertook a clinical and histopathological analysis of patients presenting with Meyerson's naevi. Eleven patients with the characteristic histological features of a Meyerson's naevus were identified over a 5-year period. Diagnostic criteria included epidermal spongiosis and a dermal inflammatory infiltrate associated with a banal junctional or compound naevus. Cases were excluded if naevus cells showed moderate to severe atypia or regression. Patients were contacted by phone and interviewed regarding their lesions. The most common clinical appearance was a solitary, pruritic, erythematous eruption encircling a pre-existing pigmented naevus. The trunk and proximal upper extremities were preferentially affected. Only one clinician listed Meyerson's naevus in the clinical differential diagnosis. All cases demonstrated a pigmented junctional or compound naevus with epidermal spongiosis, parakeratosis and a perivascular lymphohistiocytic inflammatory infiltrate with scattered eosinophils. The inflammatory infiltrate consisted almost exclusively of CD3+ lymphocytes, the majority of which were CD4+. However, a substantial number were CD8+. In all patients, the lesions cleared with excision or spontaneously, without recurrence or progression to melanoma. The aetiology of this entity remains unclear and most clinicians are unlikely to be familiar with it.     

Pigmented spindle cell naevus and its variants: distinction from melanoma

We report seven examples of pigmented spindle cell naevus (PSCN) and variants, emphasizing their histopathologic differentiation from malignant melanoma (CMM). Confusion with CMM may occur because of upward migration (pagetoid spread) of cells, prominent lateral extension of lentiginous melanocytic hyperplasia, and cytological atypia in spindle cell naevi. However, these proliferations are usually associated with a symmetrical and orderly growth pattern, confinement of the pagetoid spread to the lower epidermis, 'maturation' of the dermal component, and lack of marked cytological atypia.     

Eruptive disseminated Spitz naevus (EDSN) in a young girl of Indian origin

Eruptive disseminated Spitz naevus (EDSN) is a rare entity and has never been documented in a South‐east Asian individual (of Indian origin) previously. We report an adolescent with this condition which, to our knowledge, has only been previously reported a few times.     

Trichilemmal cyst with homogeneous blue pigmentation on dermoscopy

A 61-year-old woman was referred to our dermoscopy unit for a pigmented lesion that had been present on her left arm for 8 years. The patient did not notice any enlargement or change in colour. On dermoscopy, homogeneous blue pigmentation was seen. The lesion was excised with the pre-operative diagnosis of melanoma, blue naevus and dermatofibroma. Histopathological examination showed a trichilemmal cyst in the mid-dermis. Although homogeneous blue pigmentation on dermoscopy is the hallmark of blue naevus, it may be seen in metastatic melanoma and exceptionally in hemosiderotic and cellular types of dermatofibroma. Trichilemmal cyst should be borne in mind also in the dermoscopic differential diagnosis.     

Cutaneous collision tumour (melanocytic naevus, basal cell carcinoma, seborrhoeic keratosis): a clinical, dermoscopic and pathological case report

The association of contiguous or 'collision' tumours in the same biopsy specimen is not uncommon and is often reported in the literature. The most common association, basal cell carcinoma (BCC) and naevus, is very difficult to diagnose clinically. We describe a 38-year-old woman with a previous history of melanoma, who presented with a modified pigmented lesion of the hip that had begun to change 6 months earlier. Histologically, the lesion was a melanocytic compound naevus and a BCC with a seborrhoeic keratosis. The case was investigated clinically and by focusing on the dermoscopic features and their pathological correlates. Cutaneous collision tumours are extremely difficult to diagnose preoperatively, even with the help of dermoscopy, in particular when one of the lesions is melanocytic.     

Melanomas arising from naevi and de novo melanomas — does origin matter?

BACKGROUND: It is widely accepted that some melanomas arise from pre-existing naevi, while others appear de novo. The proportions involved and the effect of melanoma origin on prognosis is unclear. OBJECTIVES: To determine whether melanomas reported by the patient to have developed from a pre-existing naevus are associated with a better or worse prognosis compared with those arising de novo when adjusted for confounding variables. METHODS: All patients attending a dedicated melanoma screening clinic between March 1997 and March 2002 were included. The distinction between melanoma arising without any pre-existing lesion (de novo) and those derived from a pre-existing lesion (naevus melanoma) was based on patient history. We categorized patients into three groups: those who gave a history of their lesion arising within a pre-existing naevus, those in whom the melanoma developed de novo and those in whom no conclusive history could be obtained. We compared prognostic indicators between the naevus and de novo melanoma groups. RESULTS: Of 8593 patients screened, 377 had a positive diagnosis of melanoma (in situ or invasive). Of these 42% had naevus melanomas, 34% new melanomas and 24% were uncertain. Patients presenting with a melanoma arising from a pre-existing naevus had a greater Breslow thickness despite presenting sooner than the de novo group, although no significant difference in thickness was found when other prognostic factors were controlled for. CONCLUSIONS: This prospective study shows that naevi that undergo malignant change may result in melanomas that are thicker and thus potentially have a worse prognosis than de novo melanomas. Although our results were not statistically significant when other risk factors were also taken into account, it is possible that a larger study would identify a significant association.     

,,Blaue`` Variante eines Naevus spilus

Summary We report on a 29-year-old female patient with an unusual pigmented lesion of the face. Clinically the lesion looked like a pigment patch of the naevus spilus type, while histological examination revealed the presence of dermal melanocytosis and multiple common blue naevi with a discrete lentiginous component in addition. The melanocytic nature of the infiltrate was ascertained by immunohistochemistry analysis using S 100 protein and HMB 45 antibodies. We interpret this lesion as agminated blue naevi in association with lentigo simplex, an unusual variant of speckled lentiginous naevus.      

Homogeneous blue pigmentation in dermatofibroma simulating a blue naevus

A case with a pigmented skin lesion that was diagnosed as a blue naevus on clinical and dermoscopic grounds and histopathologically confirmed as a dermatofibroma is presented. By means of this case, we define dermatofibroma as a new exception for 'homogeneous blue pigmentation' on dermoscopy.     

The staining pattern of pigmented spindle cell nevi with S100A6 protein

Background: Spitz nevi typically show strong diffuse staining with S100A6, whereas staining in melanomas is commonly patchy and weak. To our knowledge, S100A6 has not been studied in pigmented spindle cell nevus (PSCN), considered by many to be a variant of Spitz nevus. Methods: Forty‐six archived PSCNs were stained with S100A6 and then categorized by predominant cell size and staining pattern. Results: Eighteen (55%) of the small cell predominant nevi showed patchy staining, eight showed diffuse staining and seven were negative for S100A6. Two predominantly large‐celled ‘PSCNs’ were diffusely positive and had many histopathological attributes of classical Spitz nevi. On review, these two cases were reclassified as Spitz nevi and excluded from the remainder of this study. Of the nevi with mixed cell size, one had no expression of S100A6. In the remaining tumors, the small cells showed patchy staining in eight (80%) and diffuse staining in two (20%). The large cells showed patchy staining in four (40%) and diffuse staining in six (60%). Conclusion: In contrast to the strong diffuse S100A6 staining typical of Spitz nevi, the small spindle cells of PSCN commonly show patchy staining or fail to stain completely. In melanocytic neoplasms composed of small spindle cells, patchy S100A6 staining should not be interpreted as evidence of supporting a diagnosis of melanoma. Puri PK, Elston CA, Tyler WB, Ferringer TC, Elston DM. The staining pattern of pigmented spindle cell nevi with S100A6 protein.     

A curious serendipitous finding: Spitz naevus combined with a syringoma

Combined tumours are often a serendipitous finding. Among the spectrum of melanocytic tumours, the co‐existence of different cell populations of the same linage is a well‐recognised phenomenon. Less commonly documented, but still well documented is the event of a melanocytic tumour in collision with an epithelial benign or malignant neoplasm. Exceptionally infrequent is however the collision of a Spitz naevus with an epithelial tumour. Here we report the clinical‐dermoscopic and histopathological findings of a Spitz naevus occurring in collision with a syringoma.     

Morphological features of Spitz naevus as observed by digital videomicroscopy

A characteristic epiluminescence pattern of pigmented epithelioid and/or spindle cell naevus, or Spitz naevus, has been described previously. The aim of this study was (i) to evaluate the characteristic morphological features both of pigmented and non-pigmented epithelioid and/or spindle cell naevi observed employing a videomicroscope, (ii) to identify their histopathological correlates and (iii) to assess the improvement in diagnostic accuracy for epithelioid and/or spindle cell naevi obtained by means of this new instrumental device. Clinical, videomicroscopic and histopathological diagnoses were performed on 26 epithelioid and/or spindle cell naevi. Moreover, the videomicroscopic pattern of each lesion was described using appropriate morphological parameters. Based on their morphological aspect detected by digital videomicroscopy, epithelioid and/or spindle cell naevi can be subdivided into three main groups: (i) darkly pigmented lesions, (ii) red or light brown ESC naevi, and (iii) lesions with dark or brown areas on a light-brown background. Whereas most epithelioid and/or spindle cell naevi of the spindle cell type belonged to the morphological group I and group 3, most epithelioid cell lesions appeared as red or light-brown coloured naevi. Finally, instrumental observation by means of a videomicroscope enabled an improvement in diagnostic accuracy with respect to the naked eye observation, with an increase in sensitivity from 15% to 58%.     

Can we use video images to triage pigmented lesions?

BACKGROUND: Advances in telemedicine permit consultations where the doctor and patient are at different sites. OBJECTIVES: To determine whether this technology could be used to triage referrals of pigmented lesions to a dermatology out-patient clinic, and thereby assist in managing the ever increasing number of lesions being referred. METHODS: When patients attended clinic, a clinical diagnosis of their pigmented lesion was made and recorded. A still image of each lesion was subsequently taken from a video camera using a PC card, and stored. These images were subsequently viewed in conjunction with the general practitioner's referral information and designated as 'warrants referral' or 'does not warrant referral'. For each lesion this decision was compared with the clinical diagnosis made during the live consultation in the clinic (the 'gold standard'). Clinical diagnoses designated as warranting referral were malignant melanoma, basal cell carcinoma, keratoacanthoma, atypical naevus and pyogenic granuloma (due to the potential clinical confusion with amelanotic melanoma). Lesions that were not considered to warrant referral included benign melanocytic naevus, seborrhoeic keratosis, dermatofibroma, congenital naevus, solar lentigo, actinic keratosis and various other benign conditions. RESULTS: In total, 819 lesions were evaluated, resulting in a mean sensitivity of 81% and specificity of 73% for the technique. CONCLUSIONS: We feel that the overall sensitivity of 81% is encouraging as regards the use of such a technique as a triage tool, but that the inability to examine the whole patient or palpate the lesions is a major drawback in the safe triage of patients with pigmented lesions.     

Symmetrisch wachsende Pigmentl?sion bei einem Jugendlichen

A 16-year-old boy with multiple nevi presented with an enlarging pigmented lesion on his right chest. Previously excised nevi were all benign. Dermoscopy of the symmetrical lesion revealed a trizonal globular-homogeneous pattern. A peripheral rim of brown globules was followed by a zone with homogeneous pigmentation. The centre of the lesion showed black dots and globules. Histopathology confirmed a dysplastic compound nevus and found no diagnostic aspects of pigmented spindle cell nevus (Reed).     

Blue naevus with satellitosis mimicking malignant melanoma

Blue naevus is an acquired benign melanocytic naevus. It is a firm, sharply defined dark blue to grey-black papule or nodule, which is likely to arise from the arrested dermal melanocytes in the dermis. In the last few years, blue naevus has attracted much attention due to the recognition of new entities and to its confusion with malignant melanoma. We report a 69-year-old man who developed a blue-black nodular lesion with satellitosis on his scalp. Although clinically it was thought to be a malignant melanoma, histopathological investigation and conservative methods such as dermatoscopy and power Doppler ultrasonography did not confirm this diagnosis. Histopathological examination excluded malignant melanoma, as there were no cellular atypia and mitotic activity in either the nodular lesion or the satellitosis. Doppler ultrasonography confirmed the benign nature of the lesion. Dermatoscopic examination showed homogeneous steel-blue pigmentation with individual blue globules, dots and some brown veils, and confirmed the histopathological diagnosis. To the best of our knowledge, our case is the third reported case of a blue naevus with satellitosis mimicking malignant melanoma.     

Epithelioid blue naevus of the genital mucosa: report of four cases

Epithelioid blue naevi are an unusual cytological variant of blue naevus that have been recently described mostly in patients with the Carney complex, although they may also occur in isolation. This variant of blue naevus is composed of melanin-laden polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive depth of dermal infiltration and, in contrast with the usual stromal changes in blue naevi, epithelioid blue naevi exhibit no dermal fibrosis. We describe four cases of epithelioid blue naevus located on the genital mucosa in four patients with no evidence of the Carney complex. Three male patients showed an epithelioid blue naevus on the mucosa of the glans penis and a female patient had a lesion of the right labium minoris. Histopathologically, the lesions consisted of entirely intradermal melanocytic naevi composed mostly of heavily pigmented epithelioid melanocytes involving the dermis of the genital mucosa. Immunohistochemically, in all cases, epithelioid melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A and MiTF antibodies.     

Subungual and periungual congenital blue naevus

Subungual pigmented lesions should raise concern about malignant melanoma. Blue naevus of the nail apparatus is a rare entity, with only ten cases described in the literature. We report a 21-year-old Hispanic woman with a slowly enlarging 1.7 × 2.3-cm subungual and periungual pigmented plaque present since birth on her right second toe. Initial biopsy was consistent with a blue naevus of the cellular type and, given the recent clinical change and periungual extension, complete excision was recommended. The entire nail unit was resected down to periosteum with prior avulsion of the nail plate. Reconstruction was performed with a full-thickness skin graft. Follow up at 1 year revealed well-healed graft and donor sites with complete return of function. We present a case of a congenital subungual and periungual blue naevus of the cellular type and review the literature on this rare presentation of a congenital blue naevus.